Marfan syndrome diagnosed during childhood: Focus on cardiac events in the French database - 24/08/14
Résumé |
Objectives |
Life expectancy of patients with Marfan syndrome has increased, due to earlier diagnosis, better familial screening, regular follow-up (FU) and prophylactic aortic surgery (PASu). Incidence of events in affected patients recognized during childhood is unknown.
Methods |
Four hundred and sixty-five patients with Marfan syndrome, diagnosed before 18 years old were included in the French multicenter database. Cardio-vascular events (death, aortic dissection or PASu) were recorded.
Results |
A cardio-vascular event occurred in 25 patients (5.4% 95CI 3.5–7.8%), including PASu (n=20, 4.3% 95CI 2.5–6.2%), aortic dissection (n=3, 0.6% 95CI 0.0–1.4%) and deaths (n=2, 0.4% 95CI 0.0–1.0%) (Fig. 1). Sixteen events (64%) occurred before 19 years old (Median 15.0, min. 2.8, interquartile 11.7–16.3; PASu n=12, deaths n=2 and dissection n=2). An aortic surgery was performed in 23 patients (4.9%, 95CI 3.0–6.9%), including a Bentall procedure with mechanical aortic valve in 10 (43.5%), a valve sparing surgery in the remaining 13 (56.5%) and a supra-coronary graft in 4 (17.4%, dissection: n=2 and PASu: n=2). Mean age at the date of PASu was 17.1±6.5 year-old. Events occurred before or at inclusion in the database in 8 patients (32.0%) (PASu n=5, dissection n=2, death n=1). Dissection was observed before inclusion in 2 patients and during pregnancy in 1 patient. Kaplan-Meier survival estimate indicates that 95% of patients remained free from events at eighteen and 78% at thirty years old.
Conclusion |
Prophylactic surgery for enlarged aorta is the main cause of cardiac events in patients with Marfan syndrome diagnosed during childhood. A quarter of them have a cardiac event before thirty years old.
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Vol 107 - N° 8-9
P. 497-498 - août 2014 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.